Retinitis pigmentosa

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Definition

Retinitis pigmentosa is an eye disease in which there is damage to the retina. The retina is the layer of tissue at the back of the inner eye. This layer converts light images to nerve signals and sends them to the brain.

Alternative Names

Causes

Retinitis pigmentosa can run in families. The disorder can be caused by several genetic defects.

The cells controlling night vision (rods) are most likely to be affected. However, in some cases, retinal cone cells are damaged the most. The main sign of the disease is the presence of dark deposits in the retina.

The main risk factor is a family history of retinitis pigmentosa. It is a rare condition affecting about 1 in 4,000 people in the United States.

Symptoms

Symptoms often first appear in childhood. However, severe vision problems do not often develop before early adulthood.

Decreased vision at night or in low light. Early signs may include having a harder time moving around in the dark.

Loss of side (peripheral) vision, causing "tunnel vision."

Loss of central vision (in advanced cases). This will affect the ability to read.

Exams and Tests

Tests to evaluate the retina:

Color vision

Exam of the retina by ophthalmoscopy after the pupils have been dilated

Treatment

There is no effective treatment for this condition. Wearing sunglasses to protect the retina from ultraviolet light may help preserve vision.

Some studies suggest that treatment with antioxidants (such as high doses of vitamin A palmitate) may slow the disease. However, taking high doses of vitamin A can cause serious liver problems. The benefit of treatment has to be weighed against risks to the liver.

Clinical trials are in progress to assess new treatments for retinitis pigmentosa, including the use of DHA, which is an omega-3 fatty acid.

Other treatments, such as microchip implants into the retina that act like a microscopic video camera, are in the early stages of development. These treatments may be useful for treating blindness associated with RP and other serious eye conditions.

A vision specialist can help you adapt to vision loss. Make regular visits to an eye care specialist, who can detect cataracts or retinal swelling. Both of these problems can be treated.

Outlook (Prognosis)

The disorder will continue to progress slowly. Complete blindness is uncommon.